Abstract

Primary amyloidosis, or light chain (AL) amyloidosis is a rare finding in the gastrointestinal (GI) tract with 1% of patients with symptomatic gastric amyloidosis. Of these, 25-45% present with bleeding. We report a case of a patient with gastric AL amyloidosis presenting with upper GI bleeding, who was also found to have amyloidosis in a transplanted kidney he received about 20 years ago. A 71 year old man with end stage renal disease from hypertension, status post deceased donor kidney transplant in 1999, and heart failure with reduced ejection fraction presented with acute decompensated heart failure. His course was complicated by new deep venous thrombosis and non-ST elevation myocardial ischemia with a peak troponin of 20. He was subsequently started on a heparin drip but developed coffee ground emesis with a nadir hemoglobin of 6.3 gm/dL (baseline of 10) requiring blood transfusions. Initial endoscopy revealed class D esophagitis, a large, friable 7 cm ulceration with heaped margins in the pyloric channel extending into the antrum with adherent clot that was successfully removed, and no stigmata of active bleeding. Biopsy of the ulcer margin demonstrated reactive inflammation with negative H. pylori. Repeat biopsy from an endoscopy performed three days later for recurrent bleeding revealed findings of amyloidosis. This sample was analyzed by mass spectrometry, confirming AL (kappa) type amyloid deposition. His echo findings revealed worsening heart failure with severe diastolic dysfunction in a restrictive pattern concerning for amyloidosis but his cardiac MRI was negative for classical cardiac amyloid. A subsequent biopsy of his transplanted kidney revealed amyloidosis by Congo red stain and sclerosing glomerulopathy, but bone marrow biopsy was normal. He was started on chemotherapy with cyclophosphamide and bortezomib, but ultimately expired four months after his diagnosis. While gastric amyloidosis is an infrequent phenomenon, its visual characteristics with mucosal friability and erythema may mimic gastric cancer and should be considered in the differential when evaluating such masses. These patients may present with GI bleeding, malabsorption, protein-losing enteropathy, or chronic GI dysmotility, and correctly diagnosing amyloidosis may alter their management and prognosis.2616_A Figure 1. Ulceration after clot removed on initial EGD2616_B Figure 2. Fungating ulceration seen on second EGD2616_C Figure 3. Amyloidosis on Congo Red Stain

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